001 Egyptian consensus-based recommendations for the diagnosis and targeted management of Kawasaki disease. An initiative by the Egyptian College of Pediatric Rheumatology

Abstract Background Kawasaki disease (kDa) is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children. It is also an important reason for long-term cardiac disease into adulthood. Rapid diagnosis and management of kDa is challenging due to the heterogeneity of the disease, yet is vital for improving outcome. To date, there are no Egyptian nationally agreed, evidence-based guidelines concerning the diagnosis and management of kDa in children. Consequently, treatment regimens vary broadly. Objectives To develop a consensus, evidence-based recommendations for the diagnosis, evaluation and management of children living with kDa. Methods This study was carried out to achieve an Egyptian expert consensus on a management strategy for kDa using Delphi technique. A multistep process strategy was adopted, which started by developing 16 key clinical questions by scientific committee according to the Patient/Population, Intervention, Comparison, Outcomes and Time (PICOT) approach. The core leadership team identified clinicians and researchers with expertise in pediatric rheumatology all over Egypt. An evidence-based, systematic, literature review was conducted to compile evidence for the kDa management. Delphi process was implemented (3-rounds) to reach a consensus. Results Twenty-five expert panel participated in the 3 rounds with response rate 100%. A total of 21 recommendations, categorized into 9 domains (Definition, disease activity, predicting the development of coronary disease, assessment and monitoring (lab, imaging), treatment (acute and after acute attack), management of resistant cases, management of complications (cardiac complications, MAS and arthritis), vaccination and long term follow up. The Agreement with the recommendations (rank 7–9) ranged from 83.6–95.7%. The Consensus was reached (i.e. ≥75% of respondents strongly agreed or agreed) on all the clinical standards. Algorithm for management has also been developed. Conclusion This was an expert, consensus recommendations for the diagnosis and treatment of kDa, based on best available evidence and expert opinion. The recommendations provided a management approach based on easy-to-use algorithm and with the support of complementary tests. The implication to policy, practice, research and advocacy to provide updated recommendations for better management of kDa.


Background
Kawasaki disease (kDa) is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children. It is also an important reason for long-term cardiac disease into adulthood. Rapid diagnosis and management of kDa is challenging due to the heterogeneity of the disease, yet is vital for improving outcome. To date, there are no Egyptian nationally agreed, evidence-based guidelines concerning the diagnosis and management of kDa in children. Consequently, treatment regimens vary broadly. Objectives To develop a consensus, evidence-based recommendations for the diagnosis, evaluation and management of children living with kDa.

Methods
This study was carried out to achieve an Egyptian expert consensus on a management strategy for kDa using Delphi technique. A multistep process strategy was adopted, which started by developing 16 key clinical questions by scientific committee according to the Patient/ Population, Intervention, Comparison, Outcomes and Time (PICOT) approach. The core leadership team identified clinicians and researchers with expertise in pediatric rheumatology all over Egypt. An evidence-based, systematic, literature review was conducted to compile evidence for the kDa management. Delphi process was implemented (3-rounds) to reach a consensus.

Results
Twenty-five expert panel participated in the 3 rounds with response rate 100%. A total of 21 recommendations, categorized into 9 domains (Definition, disease activity, predicting the development of coronary disease, assessment and monitoring (lab, imaging), treatment (acute and after acute attack), management of resistant cases, management of complications (cardiac complications, MAS and arthritis), vaccination and long term follow up. The Agreement with the recommendations (rank 7-9) ranged from 83.6-95.7%. The Consensus was reached (i.e. 75% of respondents strongly agreed or agreed) on all the clinical standards. Algorithm for management has also been developed.

Conclusion
This was an expert, consensus recommendations for the diagnosis and treatment of kDa, based on best available evidence and expert opinion. The recommendations provided a management approach based on easy-to-use algorithm and with the support of complementary tests.
The implication to policy, practice, research and advocacy: to provide updated recommendations for better management of kDa. Background Pediatric rheumatic disorders are common and result in significant impairment in the quality of life, morbidity and mortality. Tanzania with a population of over 60 million out which children and youth (0-17 years) comprise of > 50% has only one paediatric rheumatologist. Given the low number of trained experts in this sub specialty, it is expected training of medical students and residents will provide adequate training in paediatric rheumatology. This survey was aimed at determining paediatric rheumatology training among medical students and residents in Tanzania.

Methods
This was a survey conducted among senior medical undergraduate students who had completed paediatric rotations and final year residents from medical schools in Tanzania. Survey questionnaire was administered through interview to final year medical students and final year paediatric resident's medical schools, the questions asked included duration of rotation in paediatric for undergraduate students and duration of paediatric residency programs.

Results
There are 11 universities in Tanzania that offer undergraduate (UG) medical degree, and 5 of them offer residency programs for pediatrics and child health. The duration for undergraduate rotation in pediatrics varied among universities with the longest being 12 weeks and the shortest being 7 weeks. The duration for paediatric residency programs is 3 years in all the universities with an exception of one university which the program for 4 years. There were only two universities that reported to train pediatric rheumatology to undergraduate students in the country, and the topics taught to both of the universities were juvenile idiopathic arthritis. Five universities that provide undergraduate training were excluded due to absence of final year UG students. Final year residents in three out of five universities which offer paediatric residency programs reported to have trained in pediatric rheumatology. One university did not have any class session on pediatric rheumatology, and the other was excluded since there were no final year residents. Common paediatric rheumatology topics covered for residents in the three universities were juvenile idiopathic arthritis and systemic lupus erythematosus. Only one of the three universities had an extra class on dermatomyositis.

Conclusion and Recommendations
Pediatric rheumatology is not adequately taught to university trainees at both undergraduates and paediatric residency levels. There is a need to put emphasis on exposing all trainees in pediatric to catall common pediatric rheumatology conditions because of the severe shortage of rheumatology trained practitioners in Tanzania.

Introduction
Juvenile Idiopathic Arthritis (JIA) is a group of conditions in which the first sign of the disease appears before the child's 16 th birthday. The common feature is the presence of at least one arthritis of > 6 weeks' duration with no recognized aetiology. JIA affect approximately one child in 5000 before the age of 16.